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Biomedical subjects

E Uyama

Publications and source records attributed to E Uyama.

90 records · Page 5Linked to original sources
PubMed1989

Pulmonary involvement in patients with HTLV-I-associated myelopathy: increased soluble IL-2 receptors in bronchoalveolar lavage fluid.

A chronic spastic myelopathy associated with HTLV-I infection (HTLV-I-associated myelopathy: HAM) shows subclinical pulmonary involvement with bronchoalveolar T lymphocytosis. In the present study, we investigated 18 patients with HAM: five HTLV-I carriers without myelopathy and 13 normal control subjects seronegative for HTLV-I to determine if soluble IL-2 receptors (IL-2R), a marker of lymphocyte activation, were increased in serum and bronchoalveolar lavage (BAL) fluid from HAM patients. Serum IL-2R levels were significantly increased in patients with HAM as compared to normal control subjects (HAM versus control subjects: 633 +/- 395 versus 278 +/- 53 U/ml, p less than 0.01). There was also an increase of serum IL-2R levels in one of five HTLV-I carriers. BAL levels of soluble IL-2R were low but detectable in four normal control subjects and in one HTLV-I carrier. In HAM patients, however, soluble IL-2R levels in BAL fluid were remarkably elevated (173 +/- 110 U/mg of albumin) and were nearly 13 times higher on average than in serum (BAL fluid/serum ratio: 13 +/- 10). In patients with HAM, lavage IL-2R levels correlated well with the number of T lymphocytes (CD3+ cells) and CD4+ cells in BAL fluid (p less than 0.01). Furthermore, BAL lymphocytes obtained from HAM patients synthesized DNA spontaneously when cultured in vitro. These results suggest that, in HAM, T lymphocytes increased in the lung are activated locally to produce soluble IL-2R. Based on the results of this study, we conclude that immunologic mechanism(s) may play an important role in the development of pulmonary lesions in HAM patients.

Adult↗
PubMed1989

[A case of malignant rheumatoid arthritis with severe peripheral neuropathy].

A 72-year-old woman of definite type of malignant rheumatoid arthritis (MRA) with severe peripheral neuropathy. She has often noted pain of both shoulders or knee joints since some years ago. At the age of 71, she noticed numbness of the feet with pain and swelling of knee joints. She was diagnosed as definite type of rheumatoid arthritis by one podiatrist. Although she took some medications, she subsequently developed general fatigue, appetite loss, exacerbation of arthritis, drop feet and hands with prominent coldness. She was admitted to our hospital on March 22, 1985. On examination, she revealed purpura, decubitis, heart murmur, arthritis of knee joints, and fingers necrosis with skin ulcer. She had severe muscle weakness, and wasting of four limbs. Moderate impairment of all-modality sensations were noted in all extremities. Distal involvement was greater than proximal. Laboratory data during administration of prednisolone (60 mg/day) were as follows: glucose in urine, 2+; occult blood in urine, 1+; white blood cells count, 18600 with 92% polymorphonuclear leukocytes; erythrocyte sedimentation rate, 60 mm in an hour; CRP, 14.62 mg/dl (normal 0.5 greater than); RA test, 2+; RAHA, 10240; CH50, 10 U/ml (normal 32-42); C3, 37 mg/dl (normal 55-75); C4, 9 mg/dl (normal 15-28); immune complex, 4.4 micrograms/ml (normal 3.0): Chest X-ray film showed cardiomegaly (CTR, 57%). ECG disclosed atrial premature contraction, and echo cardiography suggested epicarditis with aortic valve insufficiency. 99mTc RI angiogram revealed impairment of peripheral circulation. SCV on sural nerve was not elicited. Sural nerve biopsy showed obliterans type of endoarteritis and axonal degeneration with loss of myelinated fiber.

Aged↗
PubMed1989

[Palilalia associated with progressive supranuclear palsy].

Two cases of progressive supranuclear palsy (PSP) with palilalia were presented. Case 1: A 64-year-old woman was in good health until age 62 when she noted clumsiness in walking. Subsequently, forgetfulness, abrupt falls and difficulty in swallowing developed. At the age of 63, she tended to repeat same words in conversation. On admission, neurological examination revealed mental deterioration (Hasegawa's scale 19), unsteady emotional state, supranuclear vertical gaze palsy, pseudobulbar palsy, nuchal dystonia, pyramidal tract signs and extrapyramidal signs. Although spontaneous speech production was reduced, she could answer to questions with compulsive repetition of a phrase or word. She always repeated twice or three times without stuttering, logoclonus or "palilalie aphone". Case 2: A 68-year-old, right-handed woman noted double vision since about one year ago. Subsequently, she developed slowness of voluntary movement, forgetfulness and difficulty in walking. Neurological examination showed similar signs as Case 1. She was not palilaic on examination, however we found the description of her palilalia on nurse's working records. Palilalia was not present so constantly as Case 1, but continued for about a year. We suppose that palilalia is not a rare phenomenon in PSP. Although the pathogenesis of palilalia is unknown at present, we speculate the combination of extrapyramidal sign, pseudobulbar palsy and dementia may most contribute the pathogenesis of palilalia on PSP.

Aged↗
PubMed1989

[A case of medullary infarction presented lateral medullary syndrome and respiratory arrest after ataxic respiration].

We reported a 71-year-old male with lateral medullary syndrome presented acute respiratory arrest after ataxic respiration. The patient had experienced transient diplopia repeatedly for about 2 weeks and then the developed persistent diplopia and vertigo. On the third day he was admitted to our hospital because of neurological deterioration and aspiration pneumonia. He showed left Horner's sign and double vision. And he had sensory disturbances of pain and temperature in the left face and the right side of the body, left limb ataxia and truncal ataxia. He showed dysarthria, severe dysphagia and left mild central facial paresis, but no hemiparesis. This case was clinically considered to be a typical case of left lateral medullary syndrome. When he was admitted to our hospital, he showed hypoxia with hypercapnea in spite of no history of chronic obstructive pulmonary disease. This condition was considered to be a central alveolar hypoventilation. He had two episodes of sudden-onset respiratory arrest following ataxic respiration on the 4th and 5th days, but no cardiac arrest. He was supported his respiration by mechanical ventilation until he was able to breathe spontaneously on the 29th day. The 22nd day MRI disclosed high intensity area in the left lateral and dorso-medial medulla in T2-weighted image, and this lesion was 1.5 cm in length. Therefore this case was diagnosed medullary infarction. This case developed ipsilateral facial pain in chronic stage. Pain existed around the eye and in the cheek, and pain was like toothache and unbearable like thalamic pain.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1989

[An adult case of congenital Horner's syndrome with heterochromia iridis--with special reference to alteration of Horner's sign associated with development].

It is well known that when the Horner's syndrome is congenital, a defect in pigmentation of the iris is usual; all or part of the iris remains light brown. We reported an adult case of congenital Horner's syndrome with remission and relapse of unilateral ptosis. A 25-year-old man was admitted to our hospital for ophthalmologic surgical treatment of right ptosis. According to the patient's mother, the patient was delivered with the aid of forceps at birth, and the right ptosis was observed during the first few days of his life. At 2 to 3 years of age, his parents noted lighter color of the right eye. The right ptosis was gradually improved as he grew older. However, he developed right ptosis again with left meralgia paresthesia since eighteen age. At age 25 years, he was noted to have right ptosis, right miosis (the left pupil measured 4.5 mm in diameter and the right 3.0 mm), right heterochromia iridis with pigmented iris nevi, and left meralgia paresthesia . Laboratory data of urine, blood and CSF as well as radiological studies of chest X-ray, skull X-ray, spine X-ray, brain MRI and spinal cord MRI showed unremarkable. Sweating test was intact, pharmacologic test to Horner's syndrome with 5% cocaine and 1.25% 1-epinephrine indicated that the damage was pointed to the post ganglionic sympathetic neuron. Ten patients with congenital Horner's syndrome reported in Japan since 1953 were reviewed including our case. Ten of eleven were male and Horner's sign was recorded on the left eye in 8 cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

[Rendu-Osler-Weber syndrome presented paramedian thalamic and midbrain infarcts and primary medullary hemorrhage: a case report].

We reported a 41-year-old male with paramedian thalamic and midbrain infarcts due to cerebral embolism from bilateral pulmonary arterio-venous fistula and primary medullary hemorrhage. The patient had an episode of sudden onset consciousness disturbance with left Weber's syndrome (right hemiplegia and left oculomotor palsy) and vertical gaze palsy at age of 23. He noticed numbness in the left hand and the left half body under clavicular when he had got up in a morning at age 41. He had headache and left tinnitus on second and third days, and on the 3rd and 4th days, he experienced nausea. He had severe hiccup persisting from the 6th to the 13th days. The 23rd days he was admitted to our hospital. He showed dysesthesia and paresthesia in left half body under clavicular, dysesthesia in left hand and vertical gaze palsy and convergence disturbance. MRI performed on the 18th and 24th days, disclosed hyperdense mass in T1 and T2-weighted images in dorsal site of medulla, but the 70th days MRI showed no abnormal lesions. Therefore we diagnosed the high intensity mass as primary medullary hemorrhage. Cerebral angiography showed no abnormal vasculature. Many members of his family had history of sever nasal bleeding. He had skin hemangioma and mucosal hemangioma in esophagus, stomach, colon and rectum, and bilateral pulmonary arterio-venous fistula which had been operated at age 39. His mother also had skin hemangioma and pulmonary arterio-venous fistula. Therefore this family was diagnosed Rendu-Osler-Weber syndrome (hereditary hemorrhagic telangiectasia). MRI also disclosed multiple cerebral infarctions in bilateral thalamus, left cerebral peduncle and left cerebellar hemisphere.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

[Blue-rubber-bleb-nevus syndrome presented vascular dementia and chronic DIC--a case report].

We reported a female with vascular dementia due to chronic disseminated intravascular coagulation (DIC). The patient underwent operation for skin hemangiomas three times at age 18, 27, and 38. At age 43, she presented a slowly progressive dementia. She had bluish, compressive hemangiomas of skin and upper gastrointestinal tract, and she was suspected to have hemangiomas of brain, pleura, mediastinum, liver and pancreas. The hemangiomas were diagnosed blue-rubber-bleb-nevus syndrome (BRBNS). Laboratory data revealed persistent increase of serum FDP, FDP-D, FDP-E, and D-dimer. This condition was considered chronic DIC, therefore this case was the first case of combination of BRBNS and Kasabach-Merritt syndrome (chronic DIC). Brain MRI demonstrated bilateral lesions of paraventricular deep white matter and hemangioma of left temporal lobe. Because cerebral CT showed no contrast enhancement in paraventricular lesions, the lesions were considered multiple cerebral infarctions. Central nervous system (CNS)involvement in BRBNS is rare, and most cases of CNS involvement were associated with CNS angiomata. In this case it was suggested that brain was the target organ of DIC because thrombomodulin is absent or few in the human brain, therefore vascular dementia (multiple cerebral infarctions) developed due to chronic DIC.

Adult↗
PubMedDate not supplied

Identification of a Notch3 mutation in a Japanese CADASIL family. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary disease that is characterized by recurrent stroke episodes and focal neurologic deficits progressing to pseudobulbar palsy and dementia. The causative gene is the Notch3 gene on chromosome 19, and 22 missense mutations have been identified in Caucasian patients to date. To perform mutational analysis of the Notch3 gene, we identified its exon intron boundaries and prepared sets of primers for amplification of each exon. Using these primers, we determined the Notch3 gene in a Japanese family with CADASIL symptoms and found a missense mutation (Arg133Cys) in exon 4. The mutation was heterozygous and cosegregated with the disease. Thus, the Notch3 gene is responsible for CADASIL in patients across different ethnic groups.

Dementia, Multi-Infarct↗
PubMedDate not supplied

Mutations of the notch3 gene in non-caucasian patients with suspected CADASIL syndrome.

The Notch3 gene has been recently identified as a causative gene for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). To investigate the genetic contribution of Notch mutations in familial cases with vascular leukoencephalopathy, we screened 13 patients from 11 unrelated families, which were selected on the basis of magnetic resonance imaging findings and positive family history. We identified three different missense mutations in 5 patients from 4 families. Two (Arg90Cys and Arg133Cys) are the same as previously reported in Caucasian patients, the other (Cys174Phe) is a novel mutation causing a loss of a cysteine in epidermal-growth-factor-like repeats of Notch3. These data indicate that the CADASIL Notch3 mutations were found in approximately 35% of familial cases with leukoencephalopathy, suggesting genetic heterogeneity of the disease.

Adult↗